Childhood onset neurofibromatosis type 2 can be severe and genotype dependent.We present a retrospective phenotypic analysis of all ascertained children in England 1.0). Focal cortical dysplasia occurred in 26% group 3 and 4% 2A. A total of 48% of group 3 underwent ≥1 major intervention (intracranial/spinal surgery/Bevacizumab/ radiotherapy) compared to 35% of 2A; with 23% group 3 undergoing spinal surgery (schwannoma/ependymoma/meningioma resection) compared to 4% of 2A. Mean age starting Bevacizumab was 12.7 in group 3 and 14.9 years in 2A. In conclusion, group 3 phenotype manifests earlier with greater tumour load, poorer visual outcomes and more intervention.childhood NF2, NF2, NF2 genetic severity score, paediatric NF2 genotype phenotype D Gareth Evans and Allyson Parry joint contributed for this study.

IN recent years there has been considerable interest in theories of excuse within the criminal law. These discussions have often centred on efforts to formulate a theory of excuse which would account satisfactorily for the range of currently accepted excusing conditions in the criminal law.' To some extent, the writers' almost exclusive attention to excusing conditions (i.e. those excuses which operate to relieve a defendant of criminal liability entirely such as insanity, automatism or duress) reflects the important practical and procedural divisions which exist between the substantive criminal law on the one hand and the discretions of prosecution, sentencer and Parole Board on the other. Yet excuses can operate, formally or informally, at these other levels too.2 Crucially, most writers appear to assume a clear distinction between excusing conditions and mitigating excuses (ix. those excuses taken into account by way of sentencing discretion, such as mistake of law which does not negative mens rea, most cases involving the defendant's good motive for breaking the criminal law, or provocation in crimes other than murder). The basis for this assumption is rarely articulated, and when it is, it seems unconvincing. Those writers who urge or assume a sharp distinction between excusing conditions and mitigating excuses are faced with something of a problem by the existence of " partial excuses " in the criminal law. Partial excuses fit into neither group. Although they do not operate to relieve the defendant of criminal responsibility entirely, they are taken into account by the jury, prior to the verdict. They affect sentence, sometimes drastically, but they also have the important characteristic effect of changing the category of offence with which the defendant is ultimately convicted. In English law the two leading partial excuses are provocation and diminished respon~ibility.~ Typically, the writers strive to force partial excuses into one or other of their groups, usually that of mitigating excuse. One such response is that of Professor Hart 4: " Sometimes, however, legal rules provide that the presence of a mitigating factor shall always remove the offence into a separate category carrying a lower maximum penalty. This is ' formal ' mitigation and the most prominent example of it is provocation which in English law is operative only in relation

No abstract

No abstract

Sir, Response to 'Primary surgical posterior capsulotomy during phacovitrectomy'We would like to thank Alexander and Luff 1 for their interest in our article. 2 Their described technique for primary surgical capsulotomy during phacovitrectomy is an interesting approach. We find that the ability to remove the anterior hyaloid and visualisation of the fundus during or after phacovitrectomy due to capsular opacification are rarely an issue. When it does later develop, management with Nd:YAG capsulotomy is usually straightforward. Conflict of interestThe authors declare no conflict of interest.